Dieulafoy lesions and arteriovenous malformations

I. What every physician needs to know.

Dieulafoy lesions (Figure 1) are large tortuous arterioles in the stomach that cause between 1 to 5.8% of all upper gastrointestinal bleeds. They were initially discovered in 1898 by the French surgeon Paul Georges Dieulafoy. These lesions bleed briskly and patients can present in hemorrhagic shock. The arteriole runs close to the mucosa of the stomach and does not branch into capillaries like most vessels. The arteriole can, in essence, “pop” through the mucosa and spontaneously bleed and then return to the submucosa. Histologically these vessels show no ulcerations or vasculitis. Seventy-five percent of these lesions appear at the lesser curvature of the stomach within 6 centimeters (cm) of the gastroesophageal junction, but these lesions also appear in the duodenum, colon, surgical anastomoses, and esophagus.

Figure 1.

Gross view of Dieulafoy

There is a genetic disorder that can cause multiple arteriovenous malformations (AVMs) called Osler-Weber-Rendu syndrome. It is an inherited disorder in which AVMs can occur in multiple areas of the body including the gastrointestinal (GI) tract. When a bleed does occur the therapeutic options for Dieulafoy lesions also apply to people with Osler-Weber-Rendu syndrome.

Typically the only way to diagnose this condition is with endoscopy and visualizing the bleeding vessel and/or with angiography when it is actively bleeding.

A. History Part I: Pattern Recognition:

The key symptoms are haemorrhagic shock, anemia, and recurrent hematemesis with or without melena.

B. History Part 2: Prevalence:

The lesions have been quoted as having anywhere from a 1-14% prevalence for all upper GI bleeds. There is no association with non-steroidal anti-inflammatory drugs (NSAID) or alcohol use like many other causes of upper GI bleeds. It is twice as likely to occur in men and the median age is 54. The patients typically have several comorbidities.

C. History Part 3: Competing diagnoses that can mimic Dieulafoy lesions and arteriovenous malformations

Most other causes of upper GI bleeding can mimic this process, including varices, ulcers, cancer of the upper GI system, gastritis, coagulopathy, and epistaxis.

D. Physical Examination Findings.

Vital signs can be stable or unstable and the patient can present with pallor from the anemia and, on occasion, upper abdomen pain, or they can have a benign physical examination. If the patient has Osler-Weber-Rendu syndrome then he or she might have telangiectasias and/or port-wine stains elsewhere on the body.

E. What diagnostic tests should be performed?

Typically these patients should have a complete blood count (CBC), type and screen, and coagulation studies done. The gold standard for establishing the diagnosis is endoscopy.

1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

CBC and type and screen should always be done to ensure that the patient does not need a transfusion. Additionally, coagulation testing should be done and if there is a coagulopathy, it should be reversed with vitamin K, fresh frozen plasma or both.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Angiography can be performed when the initial endoscopy cannot visualize the vessel. Angiography can help determine the source of the bleeding especially when it is in a rare place.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

A necessary evil in terms of additional tests would be repeat endoscopies. As these lesions are difficult to spot but have a high cure rate via endoscopy, sometimes repeat endoscopies are needed to visualize the vessel and to provide hemostasis. Repeat endoscopies are needed in up to 6 percent of patients. Additionally, video capsule endoscopy and double balloon enteroscopy can be used for lesions lower in the GI tract for patients with recurrent melena, especially for stable patients in the outpatient setting.

III. Default Management.

The default management begins as with all upper GI bleeds: securing the airway if needed, resuscitation and stabilization with intravenous (IV) fluids, and blood transfusion if necessary. In addition, proton pump inhibitor drips should be started to lower the gastric potential hydrogen (pH).

A. Immediate management.

Immediate management comprises taking vital signs, serial CBC testing, complete metabolic profile, coagulation testing, and type and screen. An endoscopist should be consulted as soon as possible for endoscopy. There are many endoscopic interventions that can be performed, including banding, clipping, injection of epinephrine, argon plasma electrocoagulation, and photocoagulation. Initial management has an 85% success rate and repeat endoscopies might be needed to obtain hemostasis. The next step would be for angiography and possibly embolization. Surgery is the last resort for curative treatment. In less than 5% of cases a surgeon will need to be consulted to resect the bleeding part of the stomach.

B. Physical Examination Tips to Guide Management.

Typically there are no physical examination findings to follow to look for rebleeding besides for vital signs. The patient likely will produce melanic stools for the next several days. In rare cases the patient might have a severe rebleed causing massive hematemesis.

Serial CBC or hemoglobin and hematocrits should be followed for at least the first 24 hours as these will likely help in the management of transfusions. One should remember that the hemoglobin usually lags behind the bleed, so the initial hemoglobin might not be an accurate representation of blood loss both during a brisk bleed and after having a bleed stopped.

D. Long-term management.

While hemostasis has a high cure rate, some studies quote 30 day mortality rates as high as 13% and 17 month mortality as high as 42%. Thus patients should follow-up with an endoscopist and minimize their risk factors for any recurrent upper GI bleed. This would include avoidance of NSAIDS and alcohol as well as possibly being on acid-lowering medicines such as histamine 2 receptor (H2) blockers and proton pump inhibitors.

E. Common Pitfalls and Side-Effects of Management

Common pitfalls include endoscopists mistaking concurrent gastritis and/or ulcers as the sentinel bleed.

Patients are usually placed on proton pump inhibitor drips followed by a continuous infusion at 8 milligrams (mg) an hour usually for up to 72 hours.

IV. Management with Co-Morbidities

A. Renal Insufficiency.

Desmopressin can be used to provide temporary hemostasis in a bleeding patient who is uremic.

B. Liver Insufficiency.

People with intrinsic liver disease will likely need fresh frozen plasma (FFP) or vitamin K to help reverse their coagulopathy in order to slow down the bleeding.

C. Systolic and Diastolic Heart Failure

In patients with severe heart failure one will need to watch their oxygenation status as well as their volume status to make sure that they do not become hypoxic while they are getting resuscitated.

D. Coronary Artery Disease or Peripheral Vascular Disease

The benefit of holding antiplatelet agents will need to be weighed against the risk of acute coronary syndromes. In addition, one might have a lower threshold in transfusing blood in patients with active coronary disease.

E. Diabetes or other Endocrine issues

No change in standard management.

F. Malignancy

No change in standard management.

G. Immunosuppression (HIV, chronic steroids, etc).

No change in standard management.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change in standard management.

I. Gastrointestinal or Nutrition Issues

If patient is nutritionally deplete, supplemental vitamin K might need to be given.

J. Hematologic or Coagulation Issues

Patient’s coagulopathy will need to be reversed with vitamin K and or fresh frozen plasma. In a patient anticoagulated with a Novel Anticoagulant (NOAC), supportive care is best. In the case of a patient on dabigatran (Pradaxa®), idarucizumab (Praxbind®), an antibody fragment has recently been approved in the United States for reversal.

K. Dementia or Psychiatric Illness/Treatment

No change in standard management.

V. Transitions of Care

A. Sign-out considerations While Hospitalized.

One should signout serial CBC testing during at least the first 24 hours with transfusion parameters.

B. Anticipated Length of Stay.

Most patients with an uncomplicated Dieulafoy lesion should anticipate to stay at least 2 to 3 days.

C. When is the Patient Ready for Discharge.

When the patient is hemodynamically stable and has not had active bleeding for at least 24 hours, and is able to tolerate at least a full liquid diet.

D. Arranging for Clinic Follow-up

Arranging close follow-up is paramount for these patients.

1. When should clinic follow up be arranged and with whom.

Patients should follow-up with their gastroenterologist as well as their primary care physician.

2. What tests should be conducted prior to discharge to enable best clinic first visit.

A CBC should be done on the day of discharge to document the patient’s stabilized hematocrit.

3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

A repeat CBC could be ordered; however, close follow-up on clinical symptoms are more important.

E. Placement Considerations.

None

F. Prognosis and Patient Counseling.

Patients have at least an 85% cure rate after the initial endoscopy, but they should be counselled that if there is any more upper GI bleeding to return to the emergency room immediately.